Application of convolutional neural network for differentiating ovarian thecoma-fibroma and solid ovarian cancer based on MRI.

BACKGROUND: Ovarian thecoma-fibroma and solid ovarian cancer have similar clinical and imaging features, and it is difficult for radiologists to differentiate them. Since the treatment and prognosis of them are different, accurate characterization is crucial. PURPOSE: To non-invasively differentiate ovarian thecoma-fibroma and solid ovarian cancer by convolutional neural network based on magnetic resonance imaging (MRI), and to provide the interpretability of the model. MATERIAL AND METHODS: A total of 156 tumors, including 86 ovarian thecoma-fibroma and 70 solid ovarian cancer, were split into the training set, the validation set, and the test set according to the ratio of 8:1:1 by stratified random sampling. In this study, we used four different networks, two different weight modes, two different optimizers, and four different sizes of regions of interest (ROI) to test the model performance. This process was repeated 10 times to calculate the average performance of the test set. The gradient weighted class activation mapping (Grad-CAM) was used to explain how the model makes classification decisions by visual location map. RESULTS: ResNet18, which had pre-trained weight, using Adam and one multiple ROI circumscribed rectangle, achieved best performance. The average accuracy, precision, recall, and AUC were 0.852, 0.828, 0.848, and 0.919 (P < 0.01), respectively. Grad-CAM showed areas associated with classification appeared on the edge or interior of ovarian thecoma-fibroma and the interior of solid ovarian cancer. CONCLUSION: This study shows that convolution neural network based on MRI can be helpful for radiologists in differentiating ovarian thecoma-fibroma and solid ovarian cancer.

MRI combined with clinical features to differentiate ovarian thecoma-fibroma with cystic degeneration from ovary adenofibroma.

OBJECTIVE: To explore the value of magnetic resonance imaging (MRI) and clinical features in identifying ovarian thecoma-fibroma (OTF) with cystic degeneration and ovary adenofibroma (OAF). METHODS: A total of 40 patients with OTF (OTF group) and 28 patients with OAF (OAF group) were included in this retrospective study. Univariable and multivariable analyses were performed on clinical features and MRI between the two groups, and the receiver operating characteristic (ROC) curve was plotted to estimate the optimal threshold and predictive performance. RESULTS: The OTF group had smaller cyst degeneration degree (P < .001), fewer black sponge sign (20% vs. 53.6%, P = .004), lower minimum apparent diffusion coefficient value (ADCmin) (0.986 (0.152) vs. 1.255 (0.370), P < .001), higher age (57.4 ± 14.2 vs. 44.1 ± 15.9, P = .001) and more postmenopausal women (72.5% vs. 28.6%, P < .001) than OAF. The area under the curve of MRI, clinical features and MRI combined with clinical features was 0.870, 0.841, and 0.954, respectively, and MRI combined with clinical features was significantly higher than the other two (P < .05). CONCLUSION: The cyst degeneration degree, black sponge sign, ADCmin, age and menopause were independent factors in identifying OTF with cystic degeneration and OAF. The combination of MRI and clinical features has a good effect on the identification of the two. ADVANCES IN KNOWLEDGE: This is the first time to distinguish OTF with cystic degeneration from OAF by combining MRI and clinical features. It shows the diagnostic performance of MRI, clinical features, and combination of the two. This will facilitate the discriminability and awareness of these two diseases among radiologists and gynaecologists.

Incidental FOXL2 mutated adult granulosa cell tumour of the ovary with thecoma-like foci.

We report on the incidental finding of a FOXL2 mutated adult granulosa cell tumour of the ovary with thecoma-like foci, a rare entity recently described by Jennifer N. Stall and Robert H. Young in a series of sixteen cases in 2019, displaying features differing from conventional adult granulosa cell tumour. Our aim is to specify the morphologic and molecular particularities of this presumably underrecognized finding, with a short presentation of the typical clinical context. Awareness of this rare and challenging neoplasm with indeterminate clinical course is crucial in routine diagnostics.

MR Imaging of Germ Cell and Sex Cord Stromal Tumors.

MR imaging is useful in the detection and characterization of adnexal lesions. This review discusses the clinical findings and MR imaging appearances of two types of ovarian neoplasms: germ cell and sex cord stromal tumors. The most common of these lesions, mature cystic teratomas, is characterized by the presence of bulk fat on MR imaging. Some of the other germ cell neoplasms and sex cord stromal tumors may have suggestive clinical, laboratory, or MR imaging features (eg, lipid and fibrosis) to establish a diagnosis. The ability to differentiate benign tumors from possible malignancy can aid in patient management.

Ovarian thecoma presenting with acute ovarian torsion in pregnancy; report of a rare case.

Thecoma is a commonly benign ovarian tumor of the group "Sex cord-stromal neoplasms." This group represents <5 percent of ovarian tumors. Thecoma is extremely rare in pregnancy. Here, we describe a 17-week pregnant woman presenting with acute ovarian pedicle torsion as a result of an ovarian thecoma.

Fibrothecoma in a Virgo Intacta Adolescent with Elevated Levels of CA 125 and B-hCG: A Case Report.

Ovarian fibromas are benign tumors that consist of spindle cells in bundles or storiformly arranged with collagen fibers in the stroma. Thecomas resemble theca interna ovarian cells, and there is lipid material in their cytoplasm. There is an overlap in histological and immunohistochemical characteristics of these two benign tumors, and the term "fibrothecoma" was coined to describe such cases. Their incidence is extremely rare in adolescents. The subject of our study is a 15-year-old, unmarried, virgo intacta patient who was referred to us due to profuse vaginal bleeding and the loss of consciousness. A right ovary ultrasound examination exposed the formation of a hyperechoic tumor with a diameter of 41.2 mm × 29.5 mm. Findings of cancer antigen 125 (CA 125) in the amounts of 621.1 U/mL and 142.87 mIU/mL of the B-human chorionic gonadotropin (B-hCG) serum were determined. After preoperative preparation, we operated on the patient to remove a tumor with a diameter of 37 mm × 30 mm × 22 mm, smooth outer surface, solid cross-section, and yellowish white color. The diagnosis of fibrothecoma was made based on pathohistological examination. An unusual finding of fibrothecoma in a virgo intacta adolescent with profuse vaginal bleeding and increased levels of CA 125 and B-hCG may serve as a basis for broader thinking about the pathology of juvenile bleeding.

A 40-year-old man with a rapidly growing intrascrotal tumor in the fibroma-thecoma group.

Introduction: Tumors in the fibroma-thecoma group are benign tumors, typically found in the ovaries of postmenopausal women and occasionally develop in the testes. These tumors are mostly treated with radical orchiectomy because preoperative diagnosis confirming the benign nature is difficult. Case presentation: A 40-year-old man was incidentally pointed out to have a right intrascrotal mass, measuring approximately 10 cm on computed tomography. Malignant testicular tumor was suspected based on the location and size of the tumor. The patient underwent right radical orchiectomy. Histologically, the tumor had no evidence of malignancy, and the diagnosis of tumors in the fibroma-thecoma group was made. The patient had no recurrence 8 months after surgery. Conclusion: Intrascrotal tumors in the fibroma-thecoma group are rare benign tumors and mostly treated with radical orchiectomy due to concerns about malignancies. Further investigation is needed for accurate preoperative diagnosis, and we should be aware of these rare tumors.

Resection of an Extraovarian Noniuteinized Thecoma in a 14-Year-Old Girl.

Thecomas are rare benign sex cord-stromal tumors that account for less than 1% of all ovarian tumors. They usually affect postmenopausal women and become symptomatic with abnormal bleeding. In adolescents, less than 10 cases have been reported so far, mainly with symptoms of hormonal disbalance. Extraovarian thecomas represent an even rarer entity, with only two cases described so far, none of them in the pediatric population. We report the case of a 14-year-old girl who presented with sudden-onset abdominal pain, dysuria, and fever, as well as highly elevated serum inflammation parameters. Ultrasound and magnetic resonance imaging (MRI) revealed a large, inhomogeneous pelvic mass (16 cm × 9 cm × 13 cm) with indistinct margins, suggestive of an infiltrative malignant teratoma or sarcoma. Laparoscopy confirmed a large mass of unknown origin. In contrast to the infiltrative character seen on preoperative MRI, the tumor could be easily exteriorized and resected after conversion to laparotomy. Ovaries, fallopian tubes, and uterus remained unaffected. Histopathology revealed a benign nonluteinized thecoma. The postoperative course and 19-month follow-up were uneventful.

Synchronous uterine serous carcinoma and ovarian sex cord stromal tumor (thecoma)-A rare first case report.

Synchronous endometrial and ovarian carcinoma is a rare instance and it accounts for 50 to 70% of all synchronous female genital tract tumors. However, it is very rare to find synchronous endometrial carcinoma and ovarian sex cord-stromal tumor (thecoma). The present case is a 75-year-old woman with a complaint of post-menopausal vaginal bleeding. Radiologically, the magnetic resonance imaging (MRI) pelvis revealed altered signal intensity mass in the uterus. Frozen section and routine histopathological examination were done on radical hysterectomy. Microscopically, serous carcinoma involving uterine corpus and left Fallopian tube was identified along with the unusual finding of contralateral ovarian sex cord-stromal tumor (thecoma), which was confirmed on immunohistochemical examination. It is a very rare association and is first reported in the present study after a thorough search of the published literature. Their relationship based on a high level of estrogen produced by the hyperactive ovary is controversial as serous carcinomas are less hormone-dependent.

Pelvic effusion in patients with ovarian thecoma-fibroma associated with the tumor size and plaste CA-125 level: A retrospective magnetic resonance imaging study.

PURPOSE: To survey the occurrence rate of ascites in patients with thecoma-fibroma and its potential correlation with tumor MR imaging characteristics. METHODS: A total of 40 patients with surgically proven thecoma-fibroma were enrolled in this retrospective study. We determined the tumor size, the components (solid or cystic) and their signal intensity features. Second, we identified ascites according to the fat-suppressed sagittal T2-weighted imaging sequence and divided all cases into two groups (with or without ascites). Furthermore, we explored the correlations of ascites with tumor size, tumor solidity, pathological types, patient's postmenopausal status and serum CA-125 levels by using the χ2 test. RESULTS: (1) Among the 40 cases, 15 tumors were fibromas, 15 thecomas, and 10 fibrothecomas. Nine patients (26.47%) had elevated CA-125 levels (>35.0 U/ml). (2) Thirty-one patients had ascites (77.50%), 29 of which had a small amount of ascites. Nine cases had no ascites (22.50%). (3) MRI showed a solid mass in 22 cases (55.0%), cystic mass in five cases (12.5%) and mixed solid-cystic mass in 13 cases (32.5%). The χ2 test revealed that the incidence of ascites was significantly correlated with tumor size, tumor solidity and serum CA-125 levels (P < 0.05), but not with menopause and pathological type (P > 0.05). CONCLUSION: Our data revealed that the incidence of ascites was 77.50% and was mainly correlated with tumor size and elevated CA-125 levels. These findings have potential value for improving the diagnosis and differential diagnosis of thecoma-fibroma.

The Value of MRI Findings Combined With Texture Analysis in the Differential Diagnosis of Primary Ovarian Granulosa Cell Tumors and Ovarian Thecoma-Fibrothecoma.

OBJECTIVE: This study aims to explore the value of magnetic resonance imaging (MRI) and texture analysis (TA) in the differential diagnosis of ovarian granulosa cell tumors (OGCTs) and thecoma-fibrothecoma (OTCA-FTCA). METHODS: The preoperative MRI data of 32 patients with OTCA-FTCA and 14 patients with OGCTs, confirmed by pathological examination between June 2013 and August 2020, were retrospectively analyzed. The texture data of three-dimensional MRI scans based on T2-weighted imaging and clinical and conventional MRI features were analyzed and compared between tumor types. The Mann-Whitney U-test, χ 2 test/Fisher exact test, and multivariate logistic regression analysis were used to identify differences between the OTCA-FTCA and OGCTs groups. A regression model was established by using binary logistic regression analysis, and receiver operating characteristic curve analysis was carried out to evaluate diagnostic efficiency. RESULTS: A multivariate analysis of the imaging-based features combined with TA revealed that intratumoral hemorrhage (OR = 0.037), log-sigma-20mm-3D_glszm_SmallAreaEmphasis (OR = 4.40), and log-sigma-2-0mm-3D_glszm_SmallAreaHighGrayLevelEmphasis (OR = 1.034) were independent features for discriminating between OGCTs and OTCA-FTCA (P < 0.05). An imaging-based diagnosis model, TA-based model, and combination model were established. The areas under the curve of the three models in predicting OGCTs and OTCA-FTCA were 0.935, 0.944, and 0.969, respectively; the sensitivities were 93.75, 93.75, and 96.87%, respectively; and the specificities were 85.71, 92.86, and 92.86%, respectively. The DeLong test indicated that the combination model had the highest predictive efficiency (P < 0.05), with no significant difference among the three models in differentiating between OGCTs and OTCA-FTCA (P > 0.05). CONCLUSIONS: Compared with OTCA-FTCA, intratumoral hemorrhage may be characteristic MR imaging features with OGCTs. Texture features can reflect the microheterogeneity of OGCTs and OTCA-FTCA. MRI signs and texture features can help differentiate between OGCTs and OTCA-FTCA and provide a more comprehensive and accurate basis for clinical treatment.

Immunohistochemical and Morphological Features of Bovine Granulosa Cell Tumours in Relation to Growth Pattern and Folliculogenesis.

Granulosa cell tumours (GCTs) are the most common type of bovine ovarian neoplasm. We describe the morphological and immunohistochemical features of GCTs (n = 26) and a thecoma (n = 1) using antibodies to cytokeratin AE1/AE3, vimentin, desmin, nectin-4, anti-Müllerian hormone (AMH), inhibin α, c-erbB-2 oncoprotein and proliferating cell nuclear antigen. The results indicate that, based on the expression similarity of AE1/AE3 and inhibin α, the macrofollicular growth pattern of GCTs derives from highly differentiated granulosa cells (GCs), the Graafian follicle or early atretic follicle. The trabecular growth pattern of GCTs is inferred to be derived from GCs of the atretic follicle in mature animals. AMH is a useful marker for diagnosing bovine sex cord-stromal tumours because it was expressed in all GCT cases and in the single case of thecoma.

Testicular fibroma: A case report and literature review.

Sex cord-stromal tumors are the second most common testicular tumors after germ cell tumors. They account for about 2%-5% of adult testicular tumors. Most of these tumors are benign. The most common sex cord-stromal tumor is Leydig cell tumor. In contrast, testicular fibroma is a very rare type of sex cord-stromal tumors. Histologically, testicular fibromas resemble their ovarian counterparts; however, they are much less common than ovarian fibromas. To the best of our knowledge, <50 cases of testicular fibromas are reported in the English literature. Herein, we report a rare case of testicular fibroma with acellular collagen plaque in a 51-year-old male presenting as a painless testicular mass.

Meigs syndrome with pleural effusion as initial manifestation: A case report.

BACKGROUND: Meigs syndrome is a rare neoplastic disease characterized by the triad of benign solid ovarian tumor, ascites, and pleural effusion. In postmenopausal women with pleural effusions, ascites, elevated CA-125 level, and pelvic masses, the probability of disseminated disease is high. Nevertheless, the final diagnosis is based on its histopathologic features following surgical removal of a mass lesion. Here we describe a case of Meigs syndrome with pleural effusion as the initial manifestation. CASE SUMMARY: A 52-year-old woman presented with a 2-mo history of dry cough and oppression in the chest and was admitted to our hospital due to recurrent pleural effusion and gradual worsening of dyspnea that had occurred over the previous month. Two months before admission, the patient underwent repeated chest drainage and empirical anti-tuberculosis treatment. However, the pleural fluid accumulation persisted, and the patient began to experience dyspnea on exertion leading to admission. A computed tomography scan of the chest, abdominal ultrasound, and magnetic resonance imaging confirmed the presence of right-sided pleural effusion and ascites with a right ovarian mass. Serum tumor markers showed raised CA-125. With a suspicion of a malignant tumor, the patient underwent laparoscopic excision of the ovarian mass and the final pathology was consistent with an ovarian fibrothecoma. On the seventh day postoperation, the patient had resolution of the right-sided pleural effusion. CONCLUSION: Despite the relatively high risk of malignancy when an ovarian mass associated with hydrothorax is found in a patient with elevated serum levels of CA-125, clinicians should be aware about rare benign syndromes, like Meigs, for which surgery remains the preferred treatment.

Virilization and metabolic disease in postmenopausal women related to ovarian hyperplasia and hypertecosis / [Virilización y enfermedad metabólica en mujer postmenopáusica relacionado a hiperplasia e hipertecosis ovárica]

During menopausal transition, mild clinical signs of hyperandrogenism may appear as part of the normal aging process, but the development of frank virilization suggests a specific source of androgen excess. In this context, androgen-secreting tumors at both adrenal and ovarian levels should be ruled out. We present the case of a 51-year-old postmenopausal woman with signs of 12 month period virilization, associated with personal history of type 2 diabetes and arterial hypertension, poorly managed in the past year. Laboratory tests showed elevation of serum androgen levels and hyperinsulinemia. Images were requested, revealing both enlarged homogeneous and solid ovaries, with preserved adrenal glands, which led to suspicion of a possible thecal hyperplasia of the ovarian stroma. Laparoscopic bilateral adnexectomy was performed and the pathological report confirmed the presumptive diagnosis. One month later after surgery, serum testosterone levels returned to values ​​close to spected for a postmenopausal woman. Finding the source of virilization in postmenopausal women is challenging, and they are usually associated with rare pathologies. A detailed medical history is essential to differentiate the progressive development of virilization that characterizes benign causes from the rapid progression that characterizes malignant tumors. The adequate interpretation of laboratory tests with complementary images, as well as looking for the association of pathologies causing elevated cardiovascular risk such as diabetes and hypertension are essential to establish a right diagnosis and treatment.

Durante la transición menopáusica pueden aparecer signos clínicos leves de hiperandrogenismo, como parte del proceso de envejecimiento normal, pero el desarrollo de virilización franca sugiere una fuente específica de exceso de andrógenos debiendo descartar la presencia de tumores secretores de andrógenos tanto a nivel adrenal como ovárico. Se presenta un caso de una mujer de 51 años postmenopáusica con signos de virilización de 12 meses de evolución, asociado a antecedente personal de diabetes tipo 2 e hipertensión arterial, de mal manejo en el último año. Las pruebas de laboratorio mostraron una franca elevación de los niveles de andrógeno sérico e hiperinsulinemia asociada. Las imágenes solicitadas evidenciaron ambos ovarios aumentados de tamaño de aspecto homogéneo y sólido, con glándulas adrenales de aspecto conservado, lo que hizo sospechar de una posible hiperplasia tecal del estroma ovárico. Se realizó una anexectomía bilateral por laparoscopia, cuya anatomía patológica confirmó la presunción diagnóstica. Los dosajes de testosterona sérica al mes de la cirugía retornaron a valores cercanos a la normalidad para una mujer postmenopáusica. El diagnóstico causal de virilización en mujeres posmenopáusicas es un desafío, y por lo general están asociadas con patologías poco frecuentes. Una historia clínica detallada es fundamental para diferenciar el desarrollo progresivo de virilización que caracteriza las causas benignas de la rápida progresión que caracteriza a los tumores malignos. La interpretación de pruebas correctas de laboratorio con imágenes complementarias, así como la búsqueda de antecedentes de riesgo cardiovascular como la diabetes y la hipertensión asociadas son fundamentales para establecer un correcto diagnóstico y tratamiento.

Ovarian thecoma: A very unusual cause of postmenopausal bleeding.

We describe a 67-year-old woman with postmenopausal bleeding having ceased estrogen plus progestogen therapy nine months before. Transvaginal ultrasonography showed endometrial thickening with normal ovarian appearance. Hormonal studies revealed high estradiol and inhibin B levels but normal androgens and adrenal hormones. Magnetic resonance image demonstrated a 13-mm left ovarian tumour. Hysterectomy and bilateral salpingo-oophorectomy were performed, and the pathological study revealed an 8 mm ovarian thecoma. This case illustrates a very unusual cause of postmenopausal bleeding. We suggest a study protocol and discuss the differential diagnosis of this case.

Ovarian sex cord stromal tumours: analysis of the clinical and sonographic characteristics of different histopathologic subtypes.

BACKGROUND: Ovarian sex cord stromal tumours (OSCSTs) are rare ovarian tumours and include different histopathologic subtypes. This study aimed to analyse the clinical and sonographic characteristics of different histopathologic OSCST subtypes. METHODS: A total of 63 patients with surgically proven OSCSTs were enrolled in this retrospective study to analyse their clinical and sonographic features. Ultrasound examinations and predictive models were performed before surgery. The clinical and sonographic findings were compared according to the type of OSCST based on the histopathological diagnosis. RESULTS: The mean age of 63 patients was 52.17 years (range: 17-78 years). Eighteen patients experienced irregular vaginal bleeding (28.57% 18/63), 7 patients exhibited abnormal body hair (11.11%). 2 patients (3.17%) showed an increased level of CA125, and 25 patients (39.68%, 25/63) showed an increased level of testosterone. Forty-two patients had ovarian thecoma-fibroma groups (OTFGs). Six patients had Sertoli-Leydig cell tumours (S-LCTs), 4 patients had Leydig cell tumours (LCTs), 8 patients had ovarian granulosa cell tumours (OGCTs), 2 patients had ovarian steroid cell tumours, not otherwise specified (OSCTs-NOS), and one patient had sclerosing stromal tumours (SSTs). The mean diameter of the tumour was 47.9 mm (range: 10-258 mm). Forty-seven masses were hypoechoic (74.60%). Twenty-eight masses had posterior echo attenuation, 22 masses exhibited abundant Doppler flow signals (34.92%), and one patient had ascites (1.59%). The diagnostic accuracy of the Simple Rules (SR) and the Assessment of Different NEoplasias in the adneXa (ADNEX) model in distinguishing benign and malignant OSCSTs was 44% (30/63) and 84% (53/63), respectively. The diagnostic accuracy of the SR for OTFGs, S-LCTs & LCTs & OSCTs-NOS, OGCTs, and SSTs was 47.6% (20/42), 16.67% (2/12), 100% (8/8), and 0% (0/1), respectively. The diagnostic accuracy of the ADNEX model for OTFGs, S-LCTs & LCTs & OSCTs-NOS, OGCTs, and SSTs was 93% (31/42), 58.33% (7/12), 75% (6/8), and 100% (1/1), respectively. CONCLUSIONS: OSCSTs generally appear as a solid mass on ultrasound. Posterior echo attenuation indicates an OTFG. A solid mass with abundant Doppler flow signals indicates an S-LCT, LCT, OSCT-NOS or OGCT. Current predictive models are not very effective, but symptoms, sonographic features and serum hormones are helpful for diagnosis.

Comprehensive review of imaging features of sex cord-stromal tumors of the ovary.

Sex cord-stromal tumors of the ovary (SCST) are uncommon ovarian tumors arising from sex cord and/or stromal cells of the ovaries. They may be nonfunctional and asymptomatic or functional presenting with hyperestrogenic, hyperandrogenic or cushingoid symptoms. They present in a wide age group of women, mostly in early stages and follow a nonaggressive clinical course after surgical resection. They differ from more prevalent epithelial ovarian tumors which tend to present in older women in advanced stages with poor prognosis. Some of SCSTs are associated with clinical syndromes. We will review imaging features on ultrasound, computed tomography and magnetic resonance imaging, epidemiology and clinical presentations of these tumors.

COMPLETE REMISSION OF MEDICALLY TREATED LUTEINIZED THECOMAS WITH SCLEROSING PERITONITIS.

We describe the first French patient treated for sclerosing peritonitis syndrome associated with lutheinized thecomas. At 42 years old, she had respiratory distress with increased abdominal volume. Physical examination revealed ascites, pleural effusions, and two mobile latero-uterine masses. Radiological examinations revealed bilateral ovarian masses of 10 cm. Bilateral adnexectomy was performed by laparotomy. Histological analysis concluded that there were benign luteinized thecomas. Until the 36th postoperative day, the general condition of the patient deteriorated to become critical. A second surgical procedure was attempted revealing sclerosing fibrosis preventing access to the peritoneal cavity. Subsequently, a medical treatment combining parenteral nutrition, high intravenous doses of corticosteroids, antiestrogens, colchicine and sandostatin was administered and effective allowing continuity recovery 15 months later. The clinical outcomes has been favorable at 24 month later.

Luteinized thecoma (thecomatosis) with sclerosing peritonitis: a systematic review of the literature of the last 25 years.

INTRODUCTION: Luteinized thecoma (thecomatosis) with sclerosing peritonitis (LTSP) is a very uncommon syndrome, characterized by the presence of single or bilateral ovarian thecomas and peritoneal fibrotic lesions. The disease occurs in young women and it can lead to peritoneal fibrosis and bowel obstruction. The pathogenesis of this syndrome remains still largely unknown. Surgery represents the cornerstone of treatment, but resection alone does not always allow a complete disease control. Attempts at medical treatments have been reported in recent years, but a real standard therapy has not yet been defined. AREAS COVERED: We performed a systematic review of literature, collecting all the papers that reported cases of LTSP, since its first description in 1994. We found that, in these 25 years, less than 50 cases have been described in literature. EXPERT OPINION: Along with the established role of surgery, adjuvant treatment with hormonal agents, in particular in estrogen receptor expression, seems to be a promising approach. However, more efforts must be carried out to describe treatment and outcome of new cases, improving knowledge about this rare condition.